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Anaemias Flashcards

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1
Q

What is the definition of AML?

A

Presence of at least 20% blasts in the bone marrow or peripheral blood is diagnostic of AML (malignant myeloid progenitor cells)

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2
Q

Patient with nose bleeds, tired. What should be included in your differential?

A

Leukaemia

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3
Q

Which investigations for AML?

A

FBC, Blood film, Coag screem, bone marrow, karyotype, flow cytometry

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4
Q

Define pancytopaenia

A

anaemia, leukopenia, thrombocytopaenia

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5
Q

What are typical features in presentation of leukaemias?

A

fatigue, fever, failure to thrive, pallor, petechiae, abnormal bruising and bleeding, menorrhagia, lymphadenopathy, hepatosplenomegaly

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6
Q

Differential diagnosis for petechiae?

A

A result of low plateletes

  1. Leukaemia
  2. Meningococcal septicaemia/meningitis
  3. Vasculitis
  4. Henoch schonlein purpura HSP
  5. Abuse injury (particularly in children and vulnerable adults)
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7
Q

What are the investigations for pancytopaenia in suspected leukaemia?

A

FBC, blood film, BM biopsy, CXR (lymphadenopathy), lymph node biopsy, lumbar puncture if involvement of CNS

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8
Q

What are the three types of bone marrow biopsy?

A
  1. Bone marrow aspiration (liquid sample)
  2. Bone marrow trephine (core solid sample)
  3. Bone marrow biopsy (taken from iliac crest, sample can be analysed straight away, in contrast to trephine which requires a few days for preparation)
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9
Q

Which cell is most typically affected in ALL?

A

B-lymphocytes (85%)

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10
Q

What is seen on blood film in ALL?

A

Blast cells

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11
Q

Which genetic disorder is associated with ALL?

A

Down’s syndrome

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12
Q

What is the general treatment management for leukaemias?

A

Chemotherapy and supportive management. Targeted therapy depending on mutations. Steroids (and potentially bone marrow transplant)

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13
Q

What are the two approaches with chemotherapy?

A

Induction and consolidation

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14
Q

What are the components of supportive management for leukaemias?

A

antifungal/antimicrobial prophylaxis, blood products, palliative treatment

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15
Q

What are the age peaks for ALL?

A

<5 and >45

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16
Q

Which mutations can be observed in ALL?

A

t(15:17) and t(9:22), 30% in children and 30% in adults respectively. Depending on mutation present, treatment will be modified

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17
Q

What is the time frame of treatment for ALL?

A

2-3 years

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18
Q

How should you manage leukaemia e.g. ALL, that has resulted in CNS relapse?

A

administer drugs intrathecally

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19
Q

Which life threatening conditions are associated with leukaemias?

A

Tumour lysis syndrome, leukostasis (low hypoperfusion, risk of MI etc)

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20
Q

Which FBC results would be synonymous with leukaemia diagnosis?

A

Pancytopaenia with leukocytosis

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21
Q

Which cells are abnormal in CLL?

A

Mature lymphoid lineage- B cells

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22
Q

What is Richter’s transformation and in which leukaemia is it present in?

A

CLL can transform into high-grade lymphoma, very aggressive and specific treatment

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23
Q

Which complication can arise from CLL?

A

warm autoimmune haemolytic anaemia (and immune thrombocytopaenia)

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24
Q

Which cells are seen on blood film in CLL?

A

smear/smudge cells- aged/fragile WBCs rupture and leave smudge on film during preparation

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25
In which leukaemia can flow cytometry of peripheral blood be diagnostic?
CLL, no need for bone marrow
26
Should you treat CLL ASAP?
no, treating early can be counterproductive. Wait for cytogenetic analysis to confirm type, review clinical picture and age, they might not develop problematic symptoms if left untreated. E.g. if presence of B symptoms then begin treatment
27
Which age group are affected in CLL?
>55
28
What are B symptoms?
Fever, night sweats, and weight loss- can be present in all leukaemias
29
What are the three phases that develop in CML?
chronic phase, accelerated phase, blast phase
30
Which mutation is present in CML?
t(9:22) BCR-ABL, philadelphia chromosome
31
Which targeted therapy treats CML?
Imatinib
32
Which is the most common leukaemia in adults?
CLL
33
High numbers of which cells are present in CML?
Always basophilia, often eosinophila
34
Why can some leukaemias present with leukopenia and others leukocytosis?
WCC can populate in bone marrow and not reach systemic system. Once in circulation, they can become 'sticky'
35
What are the features of cells on blood film in AML?
High % of blast cells with rods= auer rods
36
In which leukaemia is a coag screen particularly critical?
APML= acute promyelocytic anaemia
37
What is the treatment for APML?
FFP, fibrinogen, platelets, vitamin A. Easy to correct coagulopathy, however this must be done immediately due to mortality associated with hemorrhage
38
What is tumour lysis syndrome?
release of uric acid from cells destroyed by chemo, this can form crystals in the interstitial space and tubules of the kidney, causing AKI
39
Name five complications of chemotherapy?
failure, stunted growth and development in children, infections, neurotoxicity, infertility, secondary malignancy, cardiotoxicity, tumour lysis syndrome
40
Immunocompromised patient with neutropenic sepsis + infection. Which antibiotic regimen?
Escalate to IV piperacillin/tazobactam (tazocin) + (genatamicin if NEWS >7/septic shock/stem cell or organ transplant)
41
Which antibiotic is added to piperacillin + tazocin when patient is immunocompromised and is in shock?
gentamicin
42
Which type of leukaemia is most classically associated with a coagulopathy?
acute promyelocytic leukaemia (APML)
43
What is the management of APML?
fresh frozen plasma platelet transfusions cryoprecipitate or fibrinogen concentrate
44
Which translocation is involved in APML?
15+17 PML-RARA
45
What are the consequences of PML-RARA translocation??
protein stops promyelocytes from differentiating and promotes tumour-like behaviour
46
What is the emergency ORAL treatment for suspected APML
ATRA
47
What is differentiation syndrome?
side effect of ATRA, rapid differentiation of malignant promyelocytes into more mature forms, causing a potentially fatal syndrome of weight gain, fever, hypotension, pulmonary infiltrates and respiratory failure as well as third-space fluid collections such as pleural and pericardial effusions.
48
Which three investigations are required to confirm a diagnosis of APML?
peripheral blood flow cytometry bone marrow biopsy cytogenetics
49
What are three causes of pancytopaenia?
``` aplastic anaemia acute leukaemia chronic leukaemia splenomegaly drugs ```
50
Which is the most common malignancy in childhood?
ALL, shows bimodal distribution
51
What are the causes of aplastic anaemia?
``` idopathic (always use this as answer in exam if unsure!!) drugs chemical radiation exposure viral illness ```
52
Which drugs can cause aplastic anaemia?
carbamazepine, acetazolamide
53
In which condition are target cells observed?
IDA, post-splenectomy
54
In which condition are Heinz bodies observed?
denatured globin- G6PD and alpha-thalassaemia
55
In which condition are Howell-Jolly bodies observed?
Post-splenectomy, spleen normally removes DNA, sickle cell
56
In which condition is raised reticulocyte count observed?
1%= normal, raised in haemolytic anaemia
57
Name two conditions in which shistocytes are observed?
``` =fragments of RBCs haemolytic uaraemic syndrome DIC thombotic thombocytopenic purpura metalic heart valves haemolytic anaemia ```
58
Normal range for Hb in women?
120-165 g/L
59
Normal range for Hb in men?
130-180 g/L
60
Three symptoms of anaemia?
``` Fatigue Dyspnoea Palpitations Headache Faintness ```
61
Two signs of anaemia?
``` pallor conjunctival pallor tachy dyspnoea cardiac enlargement koilonichia angular chelitis atrophic glossitis brittle hair and nails ```
62
Two causes of microcytic anaemia?
``` TAILS Thalassaemia Anaemia of chronic disease IDA Lead poisonning Sideroblastic anaemia ```
63
Two causes of normocytic anaemia?
``` 3A's 2H's Acute blood loss Anaemia of chronic disease Aplastic anaemia Haemolytic anaemia Hypothyroidism (+pregnancy, renal failure) ```
64
Two causes of macrocytic anaemia?
Megaloblastic: Vit B12 or folate deficiency. Anti-folate drugs Normoblastic: alcohol, hypothyroidism, liver disease, reticulocytosis, drugs
65
Two signs of IDA?
Koilonychia angular chelitis atrophic glossitis brittle hair and nails
66
Aside from the initial blood test, name one other investigations for anaemia?
OGD colonoscopy bone marrow biopsy
67
Name a drug that can interfere with iron absorption?
PPIs- lansoprazole or omeprazole
68
Name a condition that can interfere with iron absorption?
IBD
69
Three causes of IDA?
pregnancy blood loss- mennorhagia, cancer iron deficiency- poor diet poor absorption- coeliac, crohn's
70
How is IDA determined in the lab?
TIBC= transferrin saturation
71
Patient has high value for TBC. What does this indicate?
high serum iron levels
72
Name two causes of iron overload
excess iron in diet | acute liver damage (liver=storage site for iron)
73
Two methods of improving iron levels of patient?
oral iron= ferrous sulphate iron infusion blood transfusion
74
What is pernicious anaemia?
a cause of B12 deficiency
75
Briefly explain the pathophysiology of pernicious anaemia
Parietal cells of stomach produce intrinsic factor, which is required for absorption of B12 in the ileum. Pernicious anaemia is an autoimmune condition where antibodies from against parietal cells or intrinsic factor, therefore impacting the ability to absorb B12
76
Two effects of B12 deficiency?
``` peripheral neuropathy paraesthesia loss of vibration and proprioception visual changes mood or cognitive changes ```
77
Patient with peripheral neuropathy and parasthesia. Which haematological condition should you test them for
pernicious anaemia and B12 deficiency
78
Two specific investigations for pernicious anaemia?
intrinsic factor antibody | gastric parietal cell antibody
79
How can haemolytic anaemia be divided?
inherited | acquired
80
Two examples of inherited haemolytic anaemias?
hereditary spherocytosis thalassaemia sickle cell anaemia G6PD deficiency
81
Two examples of acquired haemolytic anaemia?
autoimmune haemolytic anaemia prosthetic valve alloimmune haemolytic anaemia (tranfusions and haemolytic disease of newborn)
82
Three clinical features that result from the destruction of RBCs?
anaemia jaundice splenomegaly
83
Three investigations for haemolytic anaemia?
``` Direct Coombs test FBC Blood film- schistocytes Bilirubin- raised from destruction of RBCs Reticulocyte count ```
84
Two features of hereditary spherocytosis presentation?
jaundice gallstones splenomegaly
85
Treatment of hereditary spherocytosis?
folate splenectomy cholecystectomy
86
Name two triggers for G6PD?
infections medications fava beans
87
Two drugs that trigger G6PD?
primaquine=antimalarial sulfasalazine sulphylureas
88
What are the two types of autoimmune haemolytic anaemia
warm | cold
89
One cause of cold and one of warm type haemolytic anaemia?
warm- idiopathic | cold- lymphoma, leukaemia, SLE, HIV, EBV, drugs
90
Name one drug that causes haemolytic anaemia?
penicillin
91
Briefly explain the pathophysiology of thalassaemia
Defects in alpha and beta protein chains of haemoglobin
92
Two investigations for thalassaemia
FBC- microcytic anaemia | DNA testing
93
WHat is a common complication of thalassaemia?
iron overload- monitor ferritin
94
Two effects of iron overload?
similar to what is seen in haemochromatosis - fatigue - liver cirrhosis - heart failure - arthritis - diabetes
95
Which diet is deficient in B12?
vegetarian/vegan
96
Does intravascular haemolysis occur in warm or cold type?
cold type
97
Three complications of sickle cell anaemia?
anaemia infection risk stroke sickle cell crises
98
57 y/o lady with lemon tinge to skin and impaired vibration sense in her distal legs + fatigued. Which anaemia does she have?
pernicious anaemia
99
Patient with hypersegmented neutrophils and macrocytic anaemia. What is the likely diagnosis? Which investigation would confirm diagnosis?
Megaloblastic anaemia | Haematinics- B12/folate

Haematology (12 decks)

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